Anemiainchildhood(小兒貧血)????Tounderstandfeaturesofhematopoiesisandbloodinchildren.Tocomprehendclinicalfeatures,diagnosisandtherapyofanemia.Tounderstandthedefinition,gradedivisionandclassificationofanemiainchildren.Tomasteretiology,pathogeny,diagnosis,therapyandpreventionofnutritionalirondeficiencyanemiaandnutritionalmegaloblasticanemia.Diseaseofhematopoieticsystem?infantileanemia（1）nutritionalirondeficiencyanemia(IDA)（2）nutritionalmegaloblasticanemia?Primary/immunitythrombocytopeniaPurpura(ITP)?Leukemiahaematogenesisofchildrenhematopoiesis--producedbloodextramedullarybeforebirthandpostnatalmesoblasthepaticmedullary3-15w6w-6ms3msEmbryostage?Mesoblastichaematogenesis：3wsbegin，8wsweaken,12-15wsdisappears。?liver：8wsbegin，6monthsgraduallyweaken,erythroblast、granularcellandmegakaryocyte.Embryostage3、spleen：12wsbeginerythrocyte,granule,lymphocyte4、Haematogenesisoflymphaticorgan?1.thoracicgland:8ws2.lymphaticnodes:11wsEmbryostage5、myelo-haematopoiesis：6monsHaematogenesisfunctionemphasis，makevariouskindsofbloodcells，uniquehematogenicorganafterbirth.Haematopoiesispostnatal?1、marrow：2、extramedullary：whenrequirementofhaemopoiesisincrease，liver、spleen、lymphadenectasis，hepatomegalyandsplenomegaly,incirculatingbloodimmatureerythrocytesandgranulocytes.?PhysiologicalhaemolysisNormalnewbornshavehigherhemoglobin(HB)andhematocritlevelsandashortenedsurvivalperiodofthefetalRBCscontributestothedevelopmentofphysiologicanemia.Physiologicalhaemolysis?erythropoiesisabruptlyceaseswithonsetofrespirationatbirth,whenthearterialoxygensaturationrisestoward95%.levelsoferythropoietin(EPO)arelow.?EPOhasadecreasedhalf-lifeandanincreasedvolumeofdistributioninnewborns.AshortenedsurvivalofthefetalRBCalsocontributestothedevelopmentofphysiologicanemia.?thesizableexpansionofbloodvolumethataccompaniesrapidweightgainduringthefirst3mooflifeaddstotheneedforincreasedRBCproduction.bloodcharacteristics–ages?redbloodcells(RBC)andHbPhysiologicalhaemolysisandanemia?writebloodcells(WBC)andclassification4-6cross?Platelets150-250×109/L?bloodvolume8-10%Redbloodcell(RBC)?Termnewbornshavearedcellmassthatishigherthanatanyothertimeoflife.?anappropriateconditionforthelowoxygenenvironmentofintrauterinelife.?TheRBCcountis5.0×1012～7.0×1012,hemoglobinconcentrationisabout150～220g/Latbirth.?TheRBCandhemoglobinconcentrationinpreterminfantsareslightlylowerthanthoseinterminfants.Redbloodcell(RBC)Thewiderangeofhemoglobinconcentrationisaccountedforby:?Variationinhowrapidlytheumbilicalcordisclamped.?Aninfant'spositionafterdelivery.Ifcordclampingisdelayedandthebabyisheldlowerthanplacenta,bothhemoglobinandbloodvolumeareincreasedbyaplacentaltransfusion.ChangeofHBafterbirthHgB2001801601401201008060401d5d10d2m3m7y12yReticulocyteReticulocyte?Reticulocyteis0.04-0.06inthefirst3days.?Reticulocytedecreasesto0.005-0.015after4-7days.?Reticulocyterisesto0.02-0.08in4-6weeks.?Reticulocyteisequaltoanadult'safter5months.Whitebloodcell(WBC)?ThenormalnumberofWBCishigherininfancyandearlychildhoodthanlaterinlife.99?WBCcountis15×10～20×10atbirth.99?After6～12hours,itriseto21×10～28×10andthenbeginstodecreaseto12×109by1week.?WBCcountmaintainsabout10×109atinfantperiodandapproachadult'sWBCcountlevelby8years.Whitebloodcell(WBC)ThechangeinWBCclassificationistheproportionbetweenlymphocyteandgranulocyte.?Lymphocyteisabout30%andgranulocyteisabout65%atbirth,butthelaterlymphocytecontrarytoneutrophilegranulocytedecreases.?Theproportionbetweenlymphocyteandgranulocyteisequalat4～6daysafterbirth..Whitebloodcell(WBC)?Lymphocyteisabout60%andgranulocyteisabout35%subsequently.?Theyareequalat4~6years.?After7yearswhitecellclassificationininfantsissimilartothatinadult.ChangeofproportioninLymphocyteandGranulocyteGranulocyteLymphocyte4-6Days4-6yearsPlateletcountNormalvaluefortheplateletcountareabout150~250×109/Landvarylittlewithage.Bloodvolume?Bloodvolumeininfantsismorethaninadults.?Thenewborn'sbloodvolumeis10%ofhisweightandabout300mlonaverage.?Achild'sisabout8%~10%ofhisweight.AnemiaDefination:Anemiaisdefinedasareductionoftheredbloodcellvolumeorhemoglobinconcentrationbelowtherangeofvaluesoccurringinhealthypersons.Anemiaisanabsolutedecreaseinhematocrit,hemoglobinconcentration,ortheRBCcount.Anemiaisnotadiagnosis,butasignofunderlyingdisease.ThecriteriaofanemiaAgeHbconcentration<28days1～4months<145g/L<90g/L4～6months6months～6years6～14years<100g/L<110g/L<120g/LAnemia1.Classification1）degree:mildmoderatesevereVerysevere2）MorphologyofRBC3）Causes:lostblood,hemolytic,deficiencyofformingHbandRBCdegree)Hb(g/L)3RBC(van/mm????Mild300-40090-110Moderate200-30060-90Severe100-20030-60Verysevere<100<30Morphology?anemiawithmicrocytosisandhypochromia?Anemiawithmacrocytosis?AnemiawithnormalcytosisAnemiaMoreanemiaMCVNormal80-94Micro-hypochromia<80Macrocytosis>94microcytosis<80MCH28-32<28>32<28MCHC32-38<3232-3832-38meancorpuscularvolume(MCV),meanscorpuscularhemoglobin(MCH),meancorpuscularhemoglobinconcentration(MCHC)Causes1.lostblood:acutechronic2.hemolysisIntrinsicmembranehereditaryspherocytosisGlycolysispyruvatekinasehemoglobinsicklecell,unstableHboxidationG6PDextrinsic:immune,infection,DICCauses3.deficiencyofformingHbandRBC?deficiencyofhematopoiesissubstance?medullaryhematopoiesisdisorder(Aplasticanemia)?Theinhibitionofhaematopoiesisinducedby:InflamationChronicnephritisToxicityCancercellsinvasionbonemarrowSymptomsofanemia?Asymptomatic:particularlyiftheanemiadevelopsoveralongtime.Generalmanifestation:palloroftheskinandmucousmembranes,lethargy,malnutrition,growthretardation.liver,spleenandlymphnodesexpansion.Digestionsystem:anorexia,nauseaandconstipation.???Symptomsofanemia?Cardiovascularandrespiratorysystem:tachycardias,increasedarterypressure,wheezeandincreasedpulse.severeanemiamaycauseheartexpansionandcongestivecardiacfailure.?Nerversystem:vertigo,tinnitus,irritability,anddisordersofattention.2.DiagnosisHistory–positivemanifestation–laboratorytestsBloodsmearBMHbananysisGrowthdevelopmentnutritionnailsfairsliverspleenandlymphnotes5points:age,course,symptoms,feeding,pastmedicalhistory,familyhistoryMorphologyofRBC,reticulocytecount,WBC,plateletcount,bonemarrowcellsmear,HB,specialexamination3.Treatment??????EliminationetiologyGeneralMedicineIntravenousbloodTransplantations:BM,stemcellsOthernutritionalanemiawithmicrocytosisandhypochromiaDefinitionnutritionalirondeficiencyanemia(IDA)Hb、mostcommon、6-24ms、specialpreventionIronmetabolism?Ironcontentanddistribution:2/3oftheironispresentinHBand1/3intissueandtransportform.AdultfemalesAdultmalesnewbornContentofelementaliron(mg/kg)405075IronmetabolismIronabsorption:?Theprimaryregulatorofironhomeostasisisintestinalironabsorption.?Ironabsorptiontakesplaceprimarilyintheduodenumbytheenterocytesatthetipoftheintestinalvilla.?Ironmustpassthoughtheapicalandthethenthebasolateralmembranesofthesecellstoreachthecirculation.IronmetabolismIronstorage:?MostbodyironiscontainedinHB,withsmalleramountsboundtoferritin(鐵蛋白)andhemosiderin（含鐵血黃素）inthereticuloendothelialsystem,myoglobininmuscle,circulatingtransferring,andiron-containingenzymes.?Themajorironstoresareintheformofferritin.?Asironcontinuestoaccumulateinthecell,asecondstorageform,hemosiderinappears.IronmetabolismIroncharacteristics:?Thefetusabsorbsironfromthemotheracrosstheplacenta.?Terminfantshaveadequatereservesforthefirst4monthsoflife.?Preterminfantshavelimitedironstoresandbecauseoftheirhigherrateofgrowth,theyoutstriptheirreservesby8weeksofage.IronmetabolismIroncharacteristics:?Atbirth,becauseof“physiologicalhaemolysis”,muchironisreleasedtoplasmaandlittleironisabsorbedfromfood,?Duringthesecondstage(about2monthsold),hematopoiesisisincreasedandmoreironisabsorbedfromfood,soirondeficiencyisrareinthisstage.?After4months,developmentincrease,ironinfoodisdeficientandironstoresexhaust,somostirondeficiencyanemiaoccursin6monthsto2yearsor3yearsoldchild.causes1.inadequateironstores:preterminfant,twin2.intakeirondeficiency3.growthanddevelopmentincreasedironrequirement4.ironabsorbabnormal5.aamountofironloss:hookworminfestation,repeatedvenesection,Meckel'sdiverticulum,recurrentepistaxis(反復(fù)鼻出血).IRONpathogenesisHbmicrocytosisandhypochromiaRBCThreestageofirondeficiencyDeficiencyofironprogressesinstagesirondepletion(ID):tissueironstoresaredeleted,undernormalcondition,thiscorrelatesdirectlywithdecreaseintheferritinlever,reticulocytepercentagedecreases.Irondeficienterythropoiesis(IDE):lossofcirculatingiron.Lowserumironlessthan30ug/dl,lowtransferringsaturationand/orelevatedtotalironbindingcapacity.??Threestageofirondeficiency?irondeficiencyanemia(IDA):irondeficiencyfollowingdepletionofbothmarrowstoreandcirculatingiron.IDIDEIDAclinicalmanifestation1.generalmanifestation:mildirondeficiencyisAsymptomatic,palloroftheskinandmucousmebranesaremostevidentandlethargy,malnutrition,growthretardation.2.liverspleenandlymphnodesenlarge3.digestionsystem:anorexia（食欲差）,nausea（惡心）,constipation（便秘）.diarrheaclinicalmanifestation4.cardiovascularandrespiratorymanifestation:tachycardia,increasedarterypressure,wheeze,increasedpulse.Severeanemiamaycauseheartexpansionandcongestivecardiacfailure.5.nervoussystemmanifestation:vertigo,irritability.??clinicalmanifestation?Mainsignsmaybepalloroftheskinandmucousmembranes.?Severeanemiamaycausecongestivecardiacfailure.?IDAininfancyandearlychildhoodisassociatedwithdevelopmentaldelayandpoorgrowth.laboratorytest1.bloodsmear2.bonemarrow3.ironmetabolismInequalityofsizeoferythrocytes，smallcell，Centralolistherozoneobviouslyhypercellular,erythroidhyperplasia,thedevelopmentofcytoplasmfallsbehindnucleus.leukocytesandmegakaryocytesarenormal.Bonemarrowironstain：ferruginationgrainsintheerythocytes.NormalbonemarrowironstainIDAironstain鐵缺乏骨髓鐵染色正常骨髓鐵染色laboratorytest?ThedecreaseofHBconcentrationismorethanthedecreaseofredcellscount.?Bloodsmearrevealsthemorefeatureofmicrocyteandhypochromia.MCV＜80fl,MCH＜26pg,MCHC＜0.31.?Reticulocyteisnormalorslightlydecreases.?WBCandplateletsarenormal..BloodcountinirondeficiencyHBRBCMCVMCHCreticulocyteWBCproportionplatelet75g/L3.54×1012/L64fl18.5pg1.3%7.54×109/Lnormal254×109/L120g/L4.24×1012/L86fl32pg1.4%7.64×109/Lnormal257×109/Llaboratorytest?Bonemarrowrevealsincreasedbasophilic?normoblastandpolychromaticnormoblast.Granulocytesystemandmegakaryocytesystemarenormal.????Ironmetabolisms(血清鐵蛋白)Serumferritin(SF)Freeerythrocyteprotoporphyrin(FEP)Serumiron,totalironbindingcapacityIroninbonemarrowIronmetabolismsIronstudySerumferritin(SF)IronstoreRedbloodcellprotoporphyrin(FEP)PercentsideroblastsID↓↓NNIDE↓↓↓↓↑↓IDA↓↓↓↓↑↑↓↓SerumironNN/↓↓↓diagnosis?firstconsider---history+clinicalmanifestation+bloodsmear?Decidediagnosis---bonemarrow+ironmetabolism?Maybeseetreatmentwithiron(Thebonemarrowishypercellular,witherythroidhyperplasia,thenormoblastsmayhavescanty,andthedevelopmentofcytoplasmfallsbehindoneofnucleus.leukocytesandmegakaryocytesarenormal.)treatment????1.2.3.4.nursingfeedinggetridofetiologyironmedicineinterfusionsbloodOralironpreparation?ferrousOraladministrationofsimpleferroussaltsferroussulfate(硫酸亞鐵)ferrousgluconate（葡萄糖酸亞鐵）polysaccharidefumarateiron?Dosage:4-6mg/kgelementalironperdayOralironpreparation?Administrationtheironpriortomeals/betweentomeals.?Administrationpreparation.ascorbicacidwithiron?Therapeuticcourse:withdrawalofironpreparation6-8weeksafterhemoglobinrecovertonormallevelorwhenSF(Serumferritin)andFEP(Freeerythrocyteprotoporphyrin)isnormal.ParenteralironpreparationTobeadministeredgastrointestinalmalabsorptionintolerancepreventseffectivetherapy.onlyfororsevereoralironParenteralironpreparationAparenteralironpreparation(irondextran)isaneffectiveformofironandisusuallysafewhengiveninaproperlycalculateddose,buttheresponsetoparenteralironisnomorerapidorcompletethanthatobtainedwithproperoraladministrationofiron,unlessmalabsorptionisafactor.Withasevereanemia,immediateredbloodcelltransfusionmayadvisable,especiallyincardiacfailureorsevereinfection,butvolumeandspeedoftransfusionmustbecontrolledwell.Wemaytransfuse,severelyanemiachildrenshouldbegivenonly2-3ml/kgofpackedcellsatanyonetime.Ifthereisevidenceoffrankcongestivefailure,amodifiedexchangetransfusionusingfresh-packedRBCsshouldbeconsidered.BloodTransfusionIrontherapyNotice:3points1.Injectionironindanger2.Reaction:12-24h(irritability↓,appetite↑)---36-48h(erythroidhyperplasia)---48-72h(reticulocytosis↑)---5-7ds(peaking)2-3wstoreticulocytes3.Times:6-8wsPrevention4points—?mothermilk?feedingspecter?foodwithiron–?preterminfantNutritionalmegaloblasticanemiaFolicacidB12causesdeficiencyanemia.ofandvitaminareprimarymegaloblasticTheclinicalfeaturesincludeanemiadecreaseofredcellismorethanthatof，theHBnormal.，thevolumeofredcellislargerthanCauses????1.lessintake2.absorbabnormal3.druginteractions4.requirementincreasedPathogenesisdihydrofolatereductasefolicacidfolicacidwith4hydratevitaminB12DNA(THFA)HbverylargeRBCMegaloblasticwithLotofHb????VitaminB12isimportanceinsynthesisofnerve.?deficiencyofvitaminB12canleadtodiscordofneurologypsychology.?InthemacrocyticanemiaproducedbydeficiencyofvitaminB12,thesymptomsandsignsincludethoseofanemiaandneuropathy.?VitaminB12deficiencyneurologypsychologysymptom?Patientsdevelopademyelinatinglesionofneuronsofthespinalcolumnandcerebralcortex.?Thisconditionresultsinparesthesiasofthehandsandfeet,unsteadinessofgait,andeventuallymemorylossandpersonalitychanges.?Thereisretardofintellectiveandphysicaldevelopment.TremblingofExtremitiesorhead,hypertensionofmuscle,tendonreflexreinforcement,positiveBabinski'ssignmayappear.Clinicalmanifestation1.Generalfeatures:puffiness,poornutrition,hairyellowed,mildedema,petechia(plt↓),mucocutaneoushemorrhage.2.featureofanemia:lethargy,extramedullary3.neurologypsychology:irritability,vertigo.4.digestivesymptoms:anorexia,nausea,diarrhea.Laboratorytests1.bloodsmear2.bonemarrow3.bloodbiochemistrytests4.othersvariationinBRCshapeandsize,macrocytosis,reticulocytecountislow,nucleatedRBCsandmegaloblasticmorphologyareoftenseen,thrombocytopeniaHypercellular,Megaloblasticchanges,hypersegmentationLaboratorytests?Bloodroutineexamination:macrocyticanemia,thedecreaseofredcellcountismorethanthedecreaseofHB.MCV＞94fl,MCH＞32pg.Rreticulocyteisdecrease.WBCandplateletsarealsodecreased.?Bonemarrow:increasedbasophilicnormoblastandpolychromaticnormoblastic.Granulocyticsystemandmegakaryocytesystem:normal/lessthannormal.Laboratorytests?VitamineB12:normalserumvitaminB12levelsrangefrom200-800ng/L,B12＜12ng/LrevealsB12deficiency.?Folate:normalserumfolatelevelsrangefrom5-6ug/L,folate＜3ug/Lrevealsdeficiency.?others:LDH:serumlacticdehydrogenase(LDH)isincreaseDiagnosis?firstconsider---history+clinical??manifestation[Markedsymptomsandsignsofcentralnervoussystem.(itsupportsdefiencyofvitaminB12.)]+.bloodsmeardecidediagnosis---.bonemarrow+metabolism(TodistinguishthedeficiencyoffolicacidwiththedeficiencyofvitaminB12.)maybeseetreat